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Objectives: Given that pulmonary arterial hypertension (PAH) and chronic thromboembolic hypertension (CTEPH) are rare yet severe subtypes of pulmonary hypertension significantly impacting patients' lives, this study analyzed the total societal costs of these conditions in Finland., Methods: PAH (n = 247) and CTEPH (n = 177) patients diagnosed between 2008 and 2019 were analyzed for primary and specialty outpatient visits, emergency visits, hospitalizations, home and institutional care, sick leaves, disability pensions, and drug costs for 5 years before and after diagnosis., Results: In PAH and CTEPH, annual specialty care number of outpatient visits increased from 3.8 and 3.3 (5 years before diagnosis) to 13.8 and 9.5 one-year post-diagnosis, then decreased to 9.2 and 4.0 at 5 years post-diagnosis. Annual inpatient days rose from 2.8 and 2.7 to 16.1 and 19.7 pre-diagnosis, then fell to 10.2 and 3.5 post-diagnosis, respectively. Within 5 years post-diagnosis, in working-age 70 % PAH and 42 % CTEPH patients received disability pensions. Drug therapy accounted for most costs (67 % in PAH and 60 % in CTEPH), followed by inpatient care, disability pensions, and outpatient care. Total costs were significantly lower for CTEPH, especially after pulmonary endarterectomy. Among PAH subtypes, the highest costs were in patients with PAH associated with connective tissue diseases., Conclusions: PAH and CTEPH cause a significant economic burden on patients and society with considerable differences depending on the PAH subtype and whether the patient has undergone PEA operation or not., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Prevention of organ damage and maintenance of long-term remission are the principal goals for treatment of ANCA-associated vasculitides. This can be accomplished by early diagnosis and swift initiation of remission-inducing agents. Outcome has improved but relapses and glucocorticoid- and cyclophosphamide-related toxicity are still major concerns. For remission induction in generalized disease a combination of glucocorticoids and cyclophosphamide or rituximab is used. Rituximab is suitable especially for younger patients with fertility concerns and when cyclophosphamide avoidance otherwise is desirable. In the treatment of relapses and refractory disease, rituximab has proved effective. As maintenance treatment rituximab prevents relapses more effectively than azathioprine.

Hepatopulmonary syndrome (HPS) is characterized by the triad of liver disease, intrapulmonary vascular dilatation and arterial deoxygenation. Mediating factors are tumor necrosis factor a, endothelin 1 and nitric oxide. Typical symptom is an increase in dyspnea while in standing position, orthodeoxia. In orthodeoxia hypoxemia accentuates while the partial pressure of oxygen in arterial blood decreases by 5% or more. The blood flow distribution to the lungs is changed as the pulmonary vascular tone is altered. Diagnosis is based on the evidence of liver disease, hypoxemia and pulmonary vascular shunt detected by the so-called bubble test. Liver transplantation is currently the only efficient therapeutic option.

Acute or subactute pulmonary damage can be either idiopathic or resulting from a known cause. The most common histologic types are diffuse acute alveolar damage (DAD) and organizing pneumonia (OP). Various kinds of infection, pulmonary drug reactions or acute exacerbations of interstitial lung diseases may cause DAD, often requiring intensive care. High-resolution computed tomography (HRTT) of the lungs is the most important diagnostic examination. Surgical lung biopsy is performed for few patients.

Mesothelioma is a fatal tumor resistant to all treatment modalities for reasons that are still unresolved. Glutathione (GSH)-associated pathways are induced by oxidants and cytotoxic drugs, and they are also involved in the progression and resistance of some tumor cells in vitro. The rate-limiting enzyme in GSH biosynthesis is gamma-glutamylcysteine synthetase (gamma GCS). However, the expression of this enzyme has not been systematically investigated in malignant tumors, and there are no studies of gamma GCS in biopsy specimens of malignant mesothelioma. We investigated the immunohistochemical distribution and expression of both subunits of gamma GCS in healthy pleural mesothelium, pleural mesothelioma tumor biopsy samples (34 cases), and mesothelioma cells in culture (7 cell lines). Nonmalignant mesothelium showed no immunoreactivity for either subunit in any of the cases. The heavy (catalytic) subunit of gamma GCS was highly immunostained in 29 and weakly positive in 5 cases. High-moderate and weak immunoreactivity of the light (regulatory) subunit of gamma GCS was found in 15 and 7 tumors, respectively, whereas 12 cases showed no reactivity. There was no correlation with either catalytic or regulatory subunit expression and patient survival. There was, however, a significant correlation between the heavy chain and multidrug resistance protein (MRP) 2 (P =.048), whereas no correlation was observed between the light chain and MRP1 or MRP2. Treatment of cultured mesothelioma cells with buthionine sulfoximine (BSO), to inhibit gamma GCS, significantly potentiated cisplatin-induced cytotoxicity mainly by nonapoptotic mechanism when assessed by counting the living cells, TUNEL (terminal deoxytransferase-mediated dUTP nick-end labeling) assay, and caspase-3 cleavage. In conclusion, gamma GCS is highly positive in most cases of malignant mesothelioma and may play an important role in the primary drug resistance of this tumor in vivo., (Copyright 2002, Elsevier Science (USA). All rights reserved.)